biostudies-literatureUnknown10Plate JSulfite is a neurotoxin, which is detoxified by the molybdenum cofactor (Moco)-dependent enzyme sulfite oxidase (SOX). In humans, SOX deficiency causes the formation of the glutamate analog S-Sulfocysteine (SSC) resulting in a constant overstimulation of ionotropic glutamatergic receptors. Overstimulation leads to seizures, severe brain damage, and early childhood death. SOX deficiency may be caused either by a mutated <i>sox</i> gene or by mutations in one of the genes of the multi-step Moco biosynthesis pathway. While patients affected in the first step of Moco biosynthesis can be treated by a substitution therapy, no therapy is available for patients affected either in the second or third step of Moco biosynthesis or with isolated SOX deficiency. In the present study, we used a combinatFrontiers in pharmacology122https://www.ebi.ac.uk/biostudies/studies/S-EPMC6454129biostudies-literatureS-Sulfocysteine Induces Seizure-Like Behaviors in Zebrafish.PMC6454129Koster RWHassan AHKruse TPlate JLehne FSassen WAfalseS-Sulfocysteine Induces Seizure-Like Behaviors in Zebrafish.Sulfite is a neurotoxin, which is detoxified by the molybdenum cofactor (Moco)-dependent enzyme sulfite oxidase (SOX). In humans, SOX deficiency causes the formation of the glutamate analog S-Sulfocysteine (SSC) resulting in a constant overstimulation of ionotropic glutamatergic receptors. Overstimulation leads to seizures, severe brain damage, and early childhood death. SOX deficiency may be caused either by a mutated <i>sox</i> gene or by mutations in one of the genes of the multi-step Moco biosynthesis pathway. While patients affected in the first step of Moco biosynthesis can be treated by a substitution therapy, no therapy is available for patients affected either in the second or third step of Moco biosynthesis or with isolated SOX deficiency. In the present study, we used a combinat2019-01-01T00:00:00Z20192025-06-01T12:02:32.734Z2025-06-01T12:02:32.734ZS-EPMC64541293100110910.3389/fphar.2019.00122